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The London Cystic Fibrosis Collaboration (LCFC) has prospectively followed a clinically diagnosed cohort of infants with cystic fibrosis (CF) born in South East England since 1999 [1–4]. Over the past 20 years, the LCFC has obtained comprehensive measures of lung function and structure, including measures of ventilation inhomogeneity (lung clearance index (LCI)) and high-resolution computed tomography (HRCT) scans. By pre-school age, 73% of this cohort had LCI above the limits of normal, compared with 7% with abnormal forced expiratory volume in 0.5 seconds (FEV 0.5 ) [1]. Children with elevated LCI during pre-school years also had worse lung function at early school age [2]. The aim of this study was to investigate how LCI changes across childhood to better understand to what extent LCI results at pre-school age are an indicator of lung disease severity in adolescence.

An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter